National Marrow Donor Program


Home > Patients and Families > Disease & Treatment > About Your Disease > Inherited Immune System Disorders

Inherited Immune System Disorders

Inherited immune system disorders are diseases in which part of the body's immune system is missing or does not work. People with an immune system disorder are less able to fight infections. While these disorders are rare, there are about 100 different types, which range from mild to severe. About 50,000 people in the United States have some type of inherited immune system disorder.

Inherited immune system disorders are also called primary immune deficiency disorders. They are called "primary" because they begin in a gene that affects the immune system. Genes carry an inherited code of instructions that tells the body how to make every cell and substance in the body. (Secondary immune deficiencies are caused by something outside the body, such as a virus or chemotherapy.)

Immune system disorders and transplant

People with severe inherited immune system disorders are at high risk for life-threatening infections. For some severe disorders, the only known cure is a bone marrow or cord blood transplant (also called a BMT). To learn more, read about two of the most severe inherited immune system disorders:
Transplant has also been used to treat some other inherited immune system disorders, including:
  • Combined immunodeficiency associated with other syndromes: Ataxia telangiectasia, DiGeorge Syndrome, X-linked immunodeficiency with normal or elevated IgM, X-linked lymphoproliferative disease, autoimmune polyglandular syndrome type 1, hyperimmunoglobulin E syndrome
  • Humoral immunodeficiency: X-linked and autosomal recessive agammaglobulinemia, common variable immunodeficiency, Ig A deficiency, Ig G subclass deficiency, Ig M deficiency
  • Complement deficiencies

To find out whether transplant is a treatment option for you or your family member, talk to a doctor who is an expert in treating these disorders.

More information on inherited immune system disorders

You can get more information about inherited immune system disorders from disease-specific organizations, such as:

For other organizations that offer information and resources, see Organizations That Can Help: A Searchable Directory.

Contributing editors

Neena Kapoor, M.D., Children's Hospital of Los Angeles, Los Angeles, Calif.
Charles Peters, M.D., Children's Mercy Hospital, Kansas City, Mo.


E-mail a Friend  E-mail a Friend
Print this Page  Print this Page

Patient Teleconferences and Events



Contact Case Manager

Translated Materials
Spanish Tagalog Vietnamese
Chinese Korean  
Page last updated: February 2006

site map | glossary | editorial board | terms of use | privacy statement